How I Found the Strong

 

Yesterday we bought a house.  That is a big deal right?  Lots of stress.  And it was.  For a family like ours, it is never that easy.  We also ended up at AI with Lil for an appointment.  Don't let me be more dramatic than I need to be, this was planned.  Our doc, Dr. R could only see us that day so that is when we saw him, before settlement.  Nothing is ever easy. 

It all worked out fine and we made it through but I am still reeling a bit and will be for a while I guess.  The house is beautiful and the neighborhood is amazing.  The kids did not want to leave the new house because the were already in the basement of the house across the street playing.  We hung out in the creek out back for hours while the house was being cleaned, so much fun.  We are VERY lucky, we have a whole week to work on the new house and move things over from the old house until we make settlement on the old house.  This is HUGE.  However, now it looks like we might also be spending a couple of days in AI somewhere in there. 

I like to be right.  It feels good and it feels validating.  But sometimes I would like to be wrong.  Lil does have lesions all over her throat and they are PG lesions.  Dr. R had a good point, She may have always had these when her disease acted up she just was not able to tell us about the pain.  She was always strident when her disease was bad and we could never figure it out.  (Strident is the funny sound kids make when they breath and they are sick.)  We are assuming from this and the pain she is describing that she also has lesions farther down (and the fact that she is actually losing weight- I knew it!) She can still eat because they are in her airway.  We are going to put her under, have her scoped, have her ears looked at by ENT and also give her a colonoscopy because she is still vomiting.  Once they put any child asleep for a procedure, they like to get as much done as possible but especially a kid like Lil.  Just thinking about doing all of this scare the *&%$ out of me.  Doing a colonoscopy means doing a clean out, doing the ear check mean possibly doing tubes.  Tubes for Lil are terrifying.  Any trauma to the skin causes more lesions.  When you see the lesions on her throat and think about causing more of them it is scary.  The fear of doing the scope is the same, if we enflame her throat more we can cause more lesions.  We are going to do a 3 day course of steroids to try to calm things down before we do any of this to see if it will help.  They want to see her at baseline.  What bothers me about this is that they are not going to see the lesions.  The steroids will calm the lesions down and possibly make them go away.  The ENT wants to see them at baseline.  Also, the colonoscopy will be done when everything is calm, again.  I understand why they do this, if we are going to go in it is safest to go in when things are calm but what is the point of going in if they are not going to see anything?!?!  Dr. R sees and feels my frustrations in this.  He calls it the double edge sword.  I get it but it is so damn frustrating.  The other thing is, this is her whole life and she is only four.  And remember there were only four people (people, not people under one but people altogether) with PG I could find that had these lesions in their mouths.  The other morning I went to wake her up and she had blood all over her mouth from one of the lesions in her mouth.  It was upsetting. 

He also talked about the fact that her tonsils are big and they may want to take them out.  He said they do not just cut tonsils out anymore, they shave them down.  I said doesn't that scare you?  He said, "It terrifies me."  I don't like when he says that.  This man does organ transplants in kids.  How can anything in my kid terrify him?

What bothers me the most is that I cannot see any of it.  I cannot see what is attacking her this time.  At least last time I could see the lesions, I could see how big they were.  I knew where, how many, all of that.  This time I just have no idea.  She is in pain and uncomfortable and I have no idea.  It is so frustrating. 

I wore one of our t-shirts the other day and she said, "Mom I don't like me in that picture."  When I asked why she said, "I look mad."  I told her no, you are fierce.  And a huge smile spread across her face.  So now we make the fierce face all of the time and she loves her shirts again.  I asked Dr. R how she can have those horrible looking lesions on her tonsils and eat sour cream and onion chips or sour patch kids. It is so frustrating.  How can she be spiking fevers and have this pain but run around and play?  He said, she has been in pain her whole life.  She has figured out how to do the things she really wants to do even with her pain.  FIERCE. 

So in the midst of moving.  In the craziness of keeping my family safe and happy while getting settled in a new house and still working I have to deal with the fact that this horrible disease is now attacking my baby on the inside and it is hurting her and we have to do something about it and it is scary to do something about it and scary not to do something about it.

In the meantime I am going to play in the creek with her and let her eat green chippies and have fun.  She is loving the new house.  And she is fierce. 

Yes.... I put Rocky in my blog!  LOL!

BTW, I still have t-shirts and sweatshirts for sale if anyone wants one. 

You Are Here for A Reason

 

Having a child with a rare disease makes you feel very helpless.  I feel very helpless.  It does not help that my child refuses, almost always, to take any medication to make herself feel better.  LillyAnna is still in the midst of her flare which means continued vomiting, fevers, pain, headaches and belly pain.  I have been wondering where the lesions are and it looks like we found them.  When I mentioned to Dr. R that she was having pain in her 'throat' I thought it might be reflux.  But oxycodone would not take reflux pain away.  He wondered if she has a lesion inside her airway.  This would make a lot of sense with her current symptoms.  She has a hard time catching her breath lately and the inhalers are not working.  She coughs frequently especially when she does any kind of exercise.  She is also eating less.  So last night I did a mouth and throat check.  Sure enough I found a lesion.  This one was on her tonsil.  It was almost impossible to get a picture of it.  I got one but you can hardly see it.  I also saw what I think is one on her tongue (I see another one like it this morning) and the one in her mouth is enflamed again.  When I told Dr. R, he told me he is not surprised. 

I was. We have had such a nice couple of months.  I thought things were going so well and that the increase in Humira was going to be our ticket but I feel like all it did was fool me.  It fooled me into thinking we were safe.  But what has really been happening is that this horrible disease has done a sneak attack and decided to go incognito on us.  Instead of creating lesions where we can see them, it is creating lesions where we cannot see them... as easily.  But they hurt just the same.  And I am helpless, even more helpless now than I was before to help my baby because I cannot see what is hurting her.  I have to guess.  I have to guess.  I have to guess. 

My four year old, almost 5 year old, drinks bottles.  These bottles are probably the reason we are not in the hospital right now for dehydration or would not be in the next couple of days.  She is sitting in my lap right now drinking one, grunting in pain. It hurts to drink.  It hurts to eat.  I am wondering when she is going to stop doing both.  Or, the flare will stop and we will be ok.  I have no idea.  I have no one to ask because there is no one to ask.  There is no Infantile Idiopathic Pyoderma Gangrnosum group.  Or even one other mom that I can ask.  Lil's symptoms are all her own.  I feel overwhelmed and I feel helpless and I feel scared and my baby hurts. But she looks so healthy and she plays and she pushes through because that is what she does and it is what she always does.  They tell us it is because she has known pain her whole life.  She has known pain her whole life.  She has known pain her whole life. 

You have all heard this before.  You have heard it many times.  If you continue to follow our story you will hear it many more times because this is our life.   It is up and down.  It is in and out.  It will not change and it will not go away.  It will not change and it will not go away.  It will not change and it will not go away.

We are moving in a week.  I am so lucky to have my support group because if I did not know all of the extraordinary things those families do under extraordinary circumstances I do not think I would believe we could pull this off.  But I do know them and I have seen what they do and so I know that we can and we will get it done because that is what we do.  We get it done.  I am going to try to not worry.  We will get it done.  We will get it done.  We will get it done. 

Thank you for listening. 

Twenty-One Elephants and Still Standing

I am calling it.  We are in the middle of a flare.  Last night we gave Lil oxycodone which we have not done in months.  But she is making this grunting noise (I have told you about it) which tells us she is in pain.  I offered her the "red medicine" and at first she refused.  We do not force it.  If she refuses it we assume she does not need it.  Lil knows her pain and she knows what ''red medicine" does for her at this point.  I just put it next to her dinner plate and in 15 minutes she picked it up and took it.  The grunting stopped 20-30 minutes later.  She is still vomiting every night, spiking fevers, showing a little swelling in her fingers and she has not been the happiest child ever as you can imagine.  But she is still Lil; playing when she can, running around when she can.  The coughing is getting a little out of control and she is less likely to sit and rest than she used to be but she will rest when it gets bad.  She is getting little pustules but the band-aids are not causing a problem; it was just that one, so strange.  We start moving in 3 weeks and I am just praying this all goes smoothly.  The last thing I ever thought we would be doing, ever, if not at this point in our lives is moving.   No part of us was planning for this or thinking of it.  It will all work out in so many ways.
The other day a friend dropped off a letter telling us how inspired he and his family still are by us and our story.  They included tickets to baseball game for our family.  I cannot tell you what a pick me up it was for me.  I just cried.  I have been talking to a couple of close friends lately, one from AI, about this journey.  About how you learn to live this life but it is hard and you make it look easy because it just becomes your life.  About how everyone's journey is their own but they follow basically the same pattern.  It is so interesting to me... and empowering.  But right now I sit here listening to that beautiful little girl who 'looks so healthy' grunt in pain and it is hard. I know I have to order her meds today, get her to take them, fight with Medicaid because (once again) they are trying to get us to pay a copay.  It just goes on and on.  So all I can say to all of you is thank you all for your continued support. 

Dark Water Rising

I have not written lately.  I have not written because I felt I have asked enough of all of you over the last three years and things have been pretty quiet so I thought it was a good time to give us all a break.  Unfortunately Lil has decided that break has come to an end.  It has not come to a screeching halt but our blissful little break has ended.
Little LillyAnna has had a relatively quiet winter.  Everyone keeps asking me how she is.  I never know how to answer that question.  What I want to say is "What do you really mean, how is she compared to a normal child? or how is she for LillyAnna?"  Because those are two very different questions.  But I never do say that. I have just been saying, "She is doing pretty well actually!"  To which everyone heaves a big sigh and I can see in their eyes they have moved on to some other worry.  In reality, she is a sick little girl that looks really darn healthy.  She has a cold all winter to the point where our Nanny has created a 'friend' for her named Chompy who sits with her and eats her dirty tissues.  I think Lil actually believes it is a toy, or even more pathetically, her friend! 

She still gets pustules although, since increasing her Humira this fall, I would say she gets less.  She also has less daily pain, but the pain is still there. 
This winter we also found out she has hearing loss in both ears.  It most likely from fluid or ear wax but we need to see an ENT and figure out what to do.  Poor kid can't hear a thing though... And now we think she may have asthma.  When she was first diagnosed with PG, each time her disease acted up her airway would constrict.  For some reason, all winter she has had a strange cough that is getting worse.  The doctor is having us try an inhaler and if it does not work move on to heavier drugs.  The biggest problem is that she coughs so hard she vomits.  Dr. R does believe it is somehow associated with the PG. 
I am working part time and it is going well but I would not be able to do it without the Nanny that we have.  She knows Lil well, she takes care of our family, and she is willing to do what it takes to take care of our family.  She is willing to take 20 minutes to get Lil to take her medicine if that is what it takes.  And Lil will listen to her when she tells her to take it. No small feat with our Lil.  They have dance parties but she knows when Lil needs to rest.  They play dolls but knows when Lil can handle playing with friends. 
So life is going on but it is no normal life, we have just learned how to live it.  I have watched that happen with a few of our friends who have to live this life too.  They have learned how to live it.
Lil uses a special kind of Band-Aid because all the other ones cause sores on her skin.  A very kind couple from Golden Crown NYB ordered a whole case of them for us and we have been using them for months with no issues.  This week, for no known reason, we pulled one off and found this: 

 

 

These are the pustules.  Again, not big but there all the same and right at the injection sites.

 

 

I then noticed that the spots where we had given Lil her last couple of injections had caused small pustules.  Now they are small however this is scary.  It is scary because if you will remember, skin trauma causes lesions with Pyoderma Gangrenosum.  We have not seen an injection site cause a pustule in three years, at a time when we had no control over this disease.  They have not turned into anything but she is running random low grade fevers and not eating dinner.  She is also vomiting just about every night again.  These are not good signs.  The other thing is, the pustules are not going away.  They are not getting any bigger either which is good.  She is also in pain.  She grunts in pain all day.  I know that grunt and I know it well. First she told me it is her belly hurting then she said she was clearing her throat.  I don't think she knows what hurts, just that she is uncomfortable. 
That kid was listening to "Roar" today and said, "Hey mom!  It's our song." Then gave me a smile that just melted my heart, looked at me with eyes wiser than they should be and nodded her head.  She just gets it. 

Move Over Rover!

I have some news to share with all of you.  We will be moving in with my father in law.  Next week we will be putting our house on the market to sell and looking for a house in which to live with him.  This situation will give us more support in many ways and we cannot be more happy to share our family and space with my father in law. 
LillyAnna has been flourishing with her new Nanny.  Since increasing her Humira (and the adjustment period following that) this fall LillyAnna is having less pain.  What comes with it is that she has been sick pretty much ever since.  Dr. R says she is good enough right now that we can get her vaccines.  It is always a give and take. 
My normal fears about Lil are rearing their ugly heads with our upcoming move but I am trying to be optimistic.  I don't know how to explain that although Lil is in less pain, there is still so much of her care that takes over our lives and makes me worry about how a move will affect her and her care.  The biggest difference now is that I have her nanny now and she makes all the difference.  She loves Lilly and loves our family and takes such good care of all of us but most especially Lil.  I never felt alone in all of this because I have all of you and loving family and an amazing husband.  But having someone who is here just to take care of and focus on Lil makes a big difference.  We have always been just on one side of qualifying for a nurse but never quite there.  This way (even though we pay for it out of pocket) I feel like I have that support.  I do not think I could even consider a move like this without her.  She is already offering to help me pack and keep the house in order for showings.  But what I know is that she will always keep Lil's care first.  And that is what is most important to us...

Home Again

It is 5am and I cannot sleep, but I am home! They told me this is a side effect of the hospital stay but I have to admit insomnia is something I have been lucky enough to have avoided my whole life so it is throwing me through a loop.  I also have to say that although the nurses at the hospital were some of the best I have ever dealt with (baring those at AI of course:) the hospital stay itself was awful.  I am not good at being helpless.  The first thing they did was insert a PICC line which pretty much rendered my right arm helpless and then hooked me up to an exorbitant number of IV drips which made any movement pretty much impossible.  Does it sound like I am complaining?  Let's just say I have a new appreciation for anyone who has spent any amount of time in the hospital and my respect for them and my own daughter has increased ten fold.  I think I had a healthy respect for that in the first place.   
It would all have been worth it but I came home to a headache that rocked the house.  This is also something they said is normal, "it happens all the time" were the nurse's exact words when I called the hospital. 
I am not giving up though.  Not yet.  If I will not give up on Lil I cannot give up on myself.  So I will continue to follow doctor's orders, try to sleep and hope the headaches stay away so I can enjoy my favorite time of year.  The good news?  Lil did great while I was away... no vomiting!  Thank you God!  The twins missed me a lot.  I am so happy to be home.  Those words are not even enough to express my gratitude for being home.  Thank you all for sticking with us...

Wemberly Worried

I thought Lil had a normal stomach bug.  Why I would think she had anything normal is beyond me.  She had been vomiting in the mornings and at night (which is not her pattern of just nighttime)  and having diarrhea which is very unusual.  This lasted about 24 hours and I thought we were over it.  For once I thought it was a normal stomach virus.  Then last night she woke up with severe pain in her stomach (side), it was so bad I gave her a dose of oxycodone.  There was vomiting and that same diarrhea.  This went on for about an hour before the medicine kicked in and she finally fell asleep.  I know she was in a lot of pain if she took medicine!  She seems fine this morning.  That is my biggest concern.  If it was a stomach bug she would be down and out, not completely fine and eating well for hours or even days and then the symtoms starting up again.  I am afraid this is a reaction to the increased Humira.  Dr. B told us any side effects would not be felt until about 3 months into the increase.  That is about where we are. 

My biggest concern is that I am going into the hospital today and I am supposed to be 'resting'.  Right.  Chris is going to have to deal with all of this by himself and work full time.  I am so worried about this baby.  I know Chris and Julie can handle it but when it comes to disease stuff I need to be involved.  Maybe it is just a stomach bug that is coming and going but what if it is not?  The pattern is just strange.  It is so hard to tell....

I am all ready for my trip to the hospital.  I have my bags packed, I am hydrating myself for my own IV.  I have written notes to the kids for each day I am in, what else can I do?  I told a friend I am going to try REALLY hard to rest so I can get out of there in 4 days.  We had a good laugh about that. She said, "I think that defeats the purpose."  I have a stack of books and they give you free WIFI but it costs 5$ a day for TV.  That is just ridiculous.  And I have my Christmas cards:)  I refuse to lose my Christmas spirit.  I thank God for my wonderful husband, amazing nanny and family and friends who have reached out to support me.  Thank you.